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Medulloblastoma

Lookup NU author(s): Professor Steven CliffordORCiD, Professor David Ellison

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Abstract

Medulloblastoma (MB) comprises a biologically heterogeneous group of embryonal tumours of the cerebellum. Four subgroups of MB have been described (WNT, sonic hedgehog (SHH), Group 3 and Group 4), each of which is associated with different genetic alterations, age at onset and prognosis. These subgroups have broadly been incorporated into the WHO classification of central nervous system tumours but still need to be accounted for to appropriately tailor disease risk to therapy intensity and to target therapy to disease biology. In this Primer, the epidemiology (including MB predisposition), molecular pathogenesis and integrative diagnosis taking histomorphology, molecular genetics and imaging into account are reviewed. In addition, management strategies, which encompass surgical resection of the tumour, cranio-spinal irradiation and chemotherapy, are discussed, together with the possibility of focusing more on disease biology and robust molecularly driven patient stratification in future clinical trials.

Publication metadata

Author(s): Northcott PA, Robinson GW, Kratz CP, Mabbott DJ, Pomeroy SL, Clifford SC, Rutkowski S, Ellison DW, Malkin D, Taylor MD, Gajjar A, Pfister SM

Publication type: Review

Publication status: Published

Journal: Nature Reviews: Disease Primers

Year: 2019

Volume: 5

Issue: 1

Online publication date: 14/02/2019

Acceptance date: 02/04/2018

ISSN (electronic): 2056-676X

Publisher: NLM (Medline)

URL: https://doi.org/10.1038/s41572-019-0063-6

DOI: 10.1038/s41572-019-0063-6

PubMed id: 30765705

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